Pediatrics in Review Note to Institutions for Site Subscriptions
HOME HELP CONTACT US SUBSCRIPTIONS CME ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Full Text (PDF)
Right arrow Rapid Responses: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when Rapid Responses are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Wanek, E. A.
Right arrow Articles by Lilly, J. R.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Wanek, E. A.
Right arrow Articles by Lilly, J. R.

Biliary Atresia

Elizabeth A. Wanek MD1
Frederick M. Karrer MD2
Carlos T. Brandt MCh, PhD3
John R. Lilly MD4
 1 Instructor in Surgery, Pediatric Surgery Research Fellow, University of Colorado School of Medicine, Denver
2 Assistant Professor of Surgery, Director of Pediatric Transplantation, University of Colorado School of Medicine, Denver
3 Visiting Associate Professor of Surgery, University of Colorado School of Medicine, Denver, and Senior Lecturer in Pediatric Surgery, Pernambuco, Brazil
4 Professor and Chief, Pediatric Surgery, University of Colorado School of Medicine, Denver

Biliary atresia is a pathologic entity in which there is obliteration of some portion of the extrahepatic bile ducts. In the past, occlusion of the proximal ducts (at the liver hilus) was referred to as "noncorrectable" (Fig 1). If only the distal duct is occluded (and the proximal duct is patent), the lesion was referred to as "correctable." The distinction is academic because current treatment and prognosis are identical. The disease is panductular, ie, both extrahepatic and intrahepatic ducts are involved. Early in the disease, however, occlusion is complete only in the extrahepatic system. Without intervention, intrahepatic biliary obstruction and, subsequently, cirrhosis supervene.

In the past, except for a few cases of the correctable variant, surgical procedures were unsuccessful until Morio Kasai performed a hepatic portoenterostomy, which was first reported in English in 1968. Bile flow was effectively reestablished in both correctable and noncorrectable forms of biliary atresia. The operation was only successful when done before the patient was 4 months of age. Subsequent confirmation of Kasai's results were reported throughout the world.

ETIOLOGY/PATHOLOGY

Biliary atresia was originally thought to be a congenital malformation. Careful histopathologic examination of excised surgical specimens indicate that this is not the case; instead, the disease is a dynamic, progressive panductular sclerotic process that may continue in the intrahepatic ducts even after surgical relief of biliary obstruction.




This article has been cited by other articles:


Home page
 PediatricsHome page
P. W. Yoon, J. S. Bresee, R. S. Olney, L. M. James, and M. J. Khoury
Epidemiology of Biliary Atresia: A Population-based Study
Pediatrics, March 1, 1997; 99(3): 376 - 382.
[Abstract] [Full Text] [PDF]


HOME HELP CONTACT US SUBSCRIPTIONS CME ARCHIVE SEARCH TABLE OF CONTENTS
Pediatrics Pediatrics in Review
Copyright © 1989 by the American Academy of Pediatrics.